Granulomatosis with polyangiitis (GPA) is a syndrome of refractory vasculitis in the upper respiratory tract, lungs, kidneys, and systemic small and medium-sized arteries; adults and children have similar courses. A pediatric GPA case with a preceding bloody pericardial effusion, which caused cardiac tamponade, and bloody stool has not been reported.
A 14-year-old boy was referred for the evaluation of prolonged fever. He had chest pain and bloody stools, and diagnostic imaging showed a pericardial effusion. Immediately after admission, his systolic blood pressure decreased (85/70 mmHg), and pericardiocentesis was performed, with aspiration of approximately 500 mL of bloody pericardial fluid. Because pericardiocentesis increased the blood pressure (115/65 mmHg), the cause of the blood pressure decrease was diagnosed as cardiac tamponade. Because the pericardial fluid cytology was negative for malignant disease, as were chest MRI and gallium scintigraphy, colonoscopy was performed and showed multiple irregular-shaped aphthae from the right transverse colon to the cecum on the contralateral side of the mesenteric attachments, and a biopsy of the aphthous region showed necrotizing granulomatous inflammation. The patient also had an elevated serine proteinase 3-anti-neutrophil cytoplasmic antibody (PR3-ANCA) level (141 IU/mL) on serological examination. Head MRI showed thickening of nasal and sinus mucosa and a cystic mass in the left sphenoid sinus. Therefore, GPA was diagnosed based on these findings, and treatment with oral prednisolone (PSL) and azathioprine was started. After the treatment, the bloody stool disappeared, and there was no recurrence of pericardial effusion even after completing PSL tapering, and the PR3-ANCA level was maintained in the normal range.
Although pericarditis is a common cardiac complication of GPA, there have been no previous reports of cardiac tamponade due to pericarditis. This is the first case of a pediatric patient with cardiac and gastrointestinal complications preceding the main symptoms, including upper and lower respiratory tract and renal symptoms, although cases of GPA with bloody stools are extremely rare. In conclusion, the sequencing of measuring ANCA levels appears important assuming the vasculitic syndrome as one of differential diseases causing persistent fever and bloody stool including such as the inflammatory bowel disease.